POS0917 TRANSTHORACIC LUNG SONOGRAPHY (LUS) – USEFUL SURVEILLANCE TOOL IN SYSTEMIC SCLEROSIS ASSOCIATED INTERSTITIAL LUNG DISEASE?
Authors: M. D’orazio, A. Lackner, S. Zenz, A. Haidmayer, J. Gretler, S. Kielhauser, J. Thiel, H. P. Brezinsek, F. Fürst-Moazedi
Journal: Annals of the Rheumatic Diseases (2022)

Background The Interstitial lung disease (ILD) represent a major cause of mortality in Systemic sclerosis (SSc). Validated methods for screening and therapy monitoring of SSc-ILD are needed. In the recent literatur, the transthoracic lung sonography (LUS) emerges as a reliable tool for the early detection of lung alterations. It was recently reported that SSc-ILD-patients have thicker artefacts reflecting the pleuraline compared with SSc-patients without ILD, but it still remains unknown if the thickness of the pleuraline changes with a proper therapy response. Objectives The aim of the study was to compare the thickness of the pleuraline of SSc-ILD-patients without biological Disease Modifying Anti-Rheumatic Drugs (bDMARDS) with those successfully treated with bDMARDs in order to establish if LUS can be a reliable surveillance tool in SSc-ILD. Additionally, since our Grazer-Schema (Mycofenolat mofetil (MMF)+ rituximab (RTX) 500 mg i.v. in week 0 and 2 every 3 months) is succesfully used in our clinic since more than 10 years in patients nonresponding to methotrexate (MTX) or cyclophosphamide, we report 10 years follow-up data of 5 Patients before and after receiving that schema. Methods 29 prospectively enrolled SSc-patients were assessed according to the European League Against Rheumatism Scleroderma Trial and Research standards. Written informed consent was obtained from all patients and the study was approved by the Ethics Committee of Graz. Validated clinical scores, such as the scleroderma disease activity (SScAS) and the scleroderma disease severity scale (SScSS) were assessed. The thickness of the pleuraline was detected with LUS. The presence of ILD was assessed by high-resolution computed tomography. Twenty-five SSc-patients enrolled between 2008 and 2009, who underwent LUS with the same technical instruments and some of the same operators, were taken as a control group since they were untreated yet or were not assuming bDMARDs(1). Results Of the 29 SSc-patients (27 females/2 males) enrolled between 2019 and 2020, seventeen patients had radiographic signs of ILD (SSc-ILD+/2019-20, n=17). Until the LUS was performed, all SSc-ILD+/2019-20 showed a good clinical response to the therapy with RTX in combination with MMF. Of the 25 SSc-patients enrolled in our previous study, (n=25; 23 females/ 2 males), twelve of these patients had radiographic signs of ILD (SScILD+/2009-10, n=12); 7 were under MTX or Cyclosporin and 5 were untreated. Additionally, data collected from 5 SSc-ILD+/2009-2010 at baseline were analysed in a 10- years- follow up. As expected, SSc-ILD+ patients had a thicker pleuraline than SSc-ILD- patients. The pleura was significantly thinner in patients under MMF und RTX compared to the pleuraline of patients without bDMARDs (1,8 mm ± 0,7 Vs 0,95 ± 0,31; p &lt; 0,00). Interestingly those patients reported less frequently dry cough or dyspnoea, showing that the lower pleura thickness could be associated with a better clinical outcome. In a 10 Year follow-up after continued treatment with MMF and RTX, a significant reduction of the thickness of the pleura could be found (Mean 0,68 ± 0,2 VS 1,5 ± 0,4; p &lt; 0,00). Interestingly, such significant improvement could be found in all of these 5 patients receiving our Grazer Schema. Remarkably, the pleuraline-thickness was comparable to the thickness of the pleura of SSc-ILD-patients. This finding shows that the increase in thickness of the pleuraline can be totally reversible under treatment. Conclusion Due to the encouraging validity, reliability and simplicity of LUS, it has been increasingly considered as an excellent screening tool for SSc-ILD. Its sensitivity to reveal parenchyma and pleura changes over time in treated patients, make it also a helpful and safe methodology to follow up SSc-ILD patients. References [1]Moazedi-Fuerst, F. C. et al. Pulmonary echography in systemic sclerosis. Clinical Rheumatology (2012) doi:10.1007/s10067-012-2055-8. Disclosure of Interests None declared